Article
Different regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria.
Human molecular genetics - 1 Nov 2008
Kawamata Hibiki, Manfredi Giovanni
Abstract excerpt
The antioxidant enzyme Cu,Zn superoxide dismutase (SOD1) is predominantly localized in the cytosol, but it is also found in mitochondria. Studies in yeast suggest that apoSOD1 is imported into mitochondria and trapped inside by folding and maturation, which is facilitated by its copper chaperone for SOD1 (CCS). Here, we show that in mammalian cells, SOD1 mitochondrial localization is dictated by its folding...
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